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Global net product sales for PROCYSBI® of $16.3 million reported for second quarter 2014 Raising net sales guidance for PROCYSBI to $65 to $70 million for 2014 Company to host conference call and webcast today at 4:30 p.m. EDT

Net product sales for PROCYSBI for the second quarter were $16.3 million, representing a 34% increase over the first quarter of 2014, and $28.4 million for the six months ended June 30, 2014. PROCYSBI became commercially available in the U.S. in June 2013 and in Germany in April 2014.

As of June 30, 2014, 237 cumulative unique patients have received PROCYSBI, which compares to 199 patients at the end of the first quarter of 2014.

The launch of PROCYSBI began in Germany during April, representing the first market in the EU where the product is available to cystinosis patients.

Cash and cash equivalents as of June 30, 2014 were $58.1 million compared to $68.1 million at the end of the first quarter of 2014.

On July 1, 2014, Raptor announced a private placement of $70 million with HealthCare Royalty Partners and its affiliates. The funding includes $60 million in new convertible senior notes and an additional $10 million of funding pursuant to an amended and restated loan agreement, originally signed on December 20, 2012. Cash and cash equivalents including net proceeds from this financing were approximately $124.0 million at July 31, 2014.

In June, data from a two-year open label extension study of delayed-release cysteamine bitartrate (PROCYSBI ®) in 40 patients with nephropathic cystinosis was published in the Journal of Pediatrics. The study demonstrated that patients who took PROCYSBI for two years were able to sustain optimal cystine control in their white blood cells and preserve kidney function over the long term.

Raptor received orphan designation from the European Commission for RP103 in the treatment of Huntington's disease. RP103 also has orphan designation for Huntington's disease in the U.S.

Raptor recently appointed Françoise De Craecker to the position of senior vice president and general manager of European operations. Ms. De Craecker will be responsible for managing Raptor's European operations and executing Raptor's plans for launching PROCYSBI in select markets across Europe. Francoise has over 30 years of experience in sales, marketing and leadership positions in the pharmaceutical and medical device sectors and most recently served at Shire as the vice president and general manager for the European Region's rare disease business.

"We continue to be very pleased by the rapid adoption of PROCYSBI in the U.S. and anticipate a solid market expansion outside the U.S. starting in Germany," said Christopher M. Starr, Ph.D., Raptor's chief executive officer. "Our steady commercial build out in the EU is proceeding according to plan and we anticipate the introduction of PROCYSBI in additional EU and global country markets over the next year. In addition, we are meeting with regulatory agencies this quarter to discuss the future development activities for RP103 as a potential treatment for Huntington's disease in the U.S. and EU."

Product Features:

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